Author Affiliations: The Vascular and Birthmarks Institute of New York, Roosevelt Hospital, New York, New York (Drs Waner and Scherer and Ms Kastenbaum); and the Department of Dermatology, University Hospital Basel, Basel, Switzerland (Dr Scherer).
To study a new surgical approach to pediatric nasal hemangiomas and the need for early surgical intervention. Childhood hemangiomas are most common in the head and neck area. They can result in life-altering situations by causing airway obstruction, disfigurement, ulceration, and other adverse effects.
A retrospective study of 44 consecutive patients with hemangiomas treated in our clinic during the last 9 years. The clinical characteristics of these hemangiomas are assessed and the outcome after surgery is discussed with respect to different surgical approaches. A new modified subunit approach is introduced.
Based on the subunit principle, the incision line was modified to allow better access to all nasal subunits. The results using this technique were superior to the results using conventional incisions with respect to accessibility of the tumor, ability to trim excess skin after tumor removal, and aesthetic outcome. In contrast to reports in the literature, early surgical intervention is advocated as a result of this study.
This modified surgical technique shows superior results to the techniques used in earlier years. We strongly advocate early surgical intervention.
Infantile hemangioma is the most common benign tumor of infancy, with an estimated incidence of 4% to 10% of infants. It occurs predominantly in girls at a girl to boy ratio ranging from 2.5:1 to 4:1; however, this predominance is more pronounced (9:1) if the hemangioma is associated with PHACE(S) syndrome (posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities [sternal clefting/supraumbilical raphe]).1
Hemangiomas exhibit a typical growth pattern in that they usually proliferate by cellular hyperplasia during the first 6 to 9 months of life. The extent of proliferation is unpredictable and can be rapid and massive. The clinical growth phase is usually followed by a much longer involution phase, which does not always lead to a complete disappearance of the lesion.
Clinical appearance allows differentiation between the more common focal hemangiomas and segmental hemangiomas that involve a broad anatomic region or developmental unit. These segmental lesions exhibit a worse prognosis with more complications and a usually less satisfying aesthetic outcome.1,2 The wait-and-see medical management policy for these hemangiomas that has been widespread and justifiable during times when few therapeutic alternatives were available should now be replaced by a more active approach. The current therapeutic armamentarium has 3 components: pharmacologic,3- 7 surgical,8 and laser or cryotherapy.9- 12 Pulsed-dye laser therapy is indicated only for the treatment of very early lesions to prevent further growth or to remove residual color in an involuting hemangioma. Owing to its limited penetration depth into tissue, it is not a suitable treatment option for bulky compound hemangiomas. For each case, treatment should meet the specific needs of the patient. A multidisciplinary approach is best because this will avoid the pitfalls of a dogmatic approach dictated by the limitations of the treating physician's specialty. Unfortunately, no rigorous evidence-based studies exist to guide the decision process.
Hemangiomas involving the nose occur in approximately 15.8% of facial hemangiomas; 5.1% of the focal lesions have been shown to affect the nasal tip.2 Many of these children not only experience social ridicule from their peers but also from members of the medical profession who, in the current literature, use terms such as “harlequin nose,” “Cyrano nose,” and “Pinocchio nose”13- 15 to describe the condition. Many children also have functional problems associated with unilateral or bilateral nasal obstruction, alteration of the nasal valve, and ulceration and destruction of the nasal columella and part of the nasal septum.
Furthermore, the mass effect of a large hemangioma will permanently misshape the nose owing to the softness and pliability of the cartilaginous structures of the nasal tip at that age. While no outcome study of nasal hemangiomas exists, most authors recognize that these lesions are associated with a poor outcome.1,4,14 These factors, coupled with the cosmetically exposed location, frequently necessitate intervention.
Thomson and Lanigan16 compared 11 cases of surgically treated hemangioma with 8 cases that were conservatively managed. The surgeons used a variety of different incisions, preferring staged excision of the hemangiomas over total removal in 1 session, and they needed an average of 4 procedures per lesion. They reported delayed primary healing and in general a much worse outcome for the surgical patients than for the conservatively managed patients. The younger the patient, the worse the outcome. This article for many years formed the basis of the “no-touch” approach to these patients.
Pitanguy et al17 advocated a midline elliptical incision, which can give good functional results and preserve the nasal contours but leaves a rather obvious midline scar on the back of the nose.
Demiri et al8 reported 47 cases of facial hemangioma, 4 of them on the nasal tip (patient ages in the nasal tip cases: 20 months, 3 years, 4 years, and 7 years). Two of the patients with nasal tip involvement were treated with an inferior rhinotomy approach, and the results were reported to be very good to excellent. One of these patients needed further surgery. The 2 other nasal tip patients each needed 3 surgical procedures, 1 to remove the tumor in an open approach and 2 to reconstruct the nasal contours. Even though the authors advocate early surgical intervention, 3 of their 4 patients were older than 3 years and 1 was a 7-year-old. The authors state that the initial resection should not be overzealous to avoid causing a contour deficiency.8
Faguer et al13 suggest a transcolumellar Rethi incision combined with a rim incision on both sides to expose the alar cartilages. They treated 6 hemangiomas with good results. This approach gives good access to any hemangioma on the very front of the nasal tip and alar subunit, but it is less appropriate for larger, more complex hemangiomas because the incision cannot be extended cranially.
The same incision has been used by McCarthy et al15 in 22 children older than 3 years. These authors emphasized the need to adjust the domes by means of a mattress suture to restore nasal projection. The removal of excess skin was achieved through a central wedge excision with vertical closure and a resulting obvious scar on the nasal tip and columella. McCarthy et al15 recommend avoiding this wedge excision if possible.
Jackson and Sosa18 propose an open rhinoplasty approach and suggest that the surplus skin not be removed but rather left in place to allow for contraction, which may take many months. The disadvantage to this approach is that a lot of skin altered by the hemangioma itself or by prior treatment attempts is left behind.
A very recent retrospective analysis by Burgos et al19 advocates surgical excision of nasal tip hemangiomas before age 3 years to avoid psychological distress and cosmetic defects.
The present study deals with the special entity of nasal hemangiomas, a new surgical approach, and the need for early surgical intervention in these children.
Forty-four consecutive infants (31 girls and 13 boys) who were treated in our clinic between 1996 and 2005 were enrolled in this retrospective study. All of them had isolated infantile nasal hemangiomas of various sizes that involved at least 1 of the following subunits: nasal tip, columella, or alar subunit (modified subunit principle, first introduced by Burget and Menick20). Children with hemangiomas predominantly involving the nasal dorsum, sidewall, or cheek were not included in the study because the surgical procedure to correct these would require a completely different approach.
The diagnosis of hemangiomas was always clinical and was confirmed histologically in all cases. The patient's characteristics are summarized in the Table. The location of each hemangioma was determined from clinical photographs and/or the patient's chart by 2 independent investigators (J.K. and K.S.).
Incisions used for surgical removal included the standard external rhinoplasty, an elliptical midline incision, and a modified subunit incision that had been devised for this purpose. The external rhinoplasty incision was chosen for small hemangiomas with a very frontal localization on the nasal tip and columella with or without a small alar part because this approach does not allow easy access to the nasal dorsum and the lateral alar subunit. We did not choose the external rhinoplasty incision for large hemangiomas because the excess skin can only be trimmed in 1 direction with this incision. If (1) full exposure of the lateral nasal cartilages was needed, (2) the hemangioma involved the nasal dorsum, (3) excision of a large tumor bulk was required, and/or (4) a large portion of the hemangioma involved the alar region, a modified subunit incision was more advantageous. The modified subunit approach also allows tailoring of excess skin after removal of a large tumor bulk in 3 dimensions, and it respects the soft triangle. The elliptical midline incision gives easy access to the nasal dorsum; however, preparation in the more lateral alar regions and the columella is not as clearly defined as with the modified subunit incision. The midline scar proved to be more visible than a scar hidden in the lateral shadow of the alar crease.
The final outcome was graded on a purely subjective scale by the same investigators who determined the locations of the hemangiomas, independently using pictures of the postoperative result. The final outcome was evaluated for each patient based on the general appearance of the area operated on, reduction in the volume of the tumor, correction of functional impairment, improvement of skin texture, and cosmetic appearance. The satisfaction of the patients or their parents was also considered.
The outcome was considered “very good” if the nasal contours were normal and the scar was almost invisible. A “good” outcome showed almost normal contours but a clearly visible scar. The outcome was considered to be an “improvement” over the presurgical condition if the hemangioma had been removed completely or almost completely but the nasal contours were still disfigured. If the nose was still severely disfigured and a significant amount of hemangioma was left behind, the outcome was considered “unsatisfactory.” Patients who had “improvement” or “unsatisfactory” results mostly had to undergo further surgical procedures.
The incision we describe is made up of several components. A midcolumellar incision is created and carried up medially to and above the soft triangles, taking care not to disrupt the soft triangles, into the nasal alar creases. From here, the incision can be continued up one or both paranasal creases if needed (Figure 1). The length and extent of the incision can be tailored to the location and extent of the lesion. A hemangioma involving predominantly the columella and inferior aspect of the nasal tip would require only minimal extension up the alar creases (Figure 2). A predominantly unilateral lesion should be approached through an asymmetric incision with more exposure on the side of the lesion (Figure 3 and Figure 4).
Schematic frontal (A) and lateral (B) drawings of the modified subunit incision. Solid lines indicate the usual lines of incision; dotted lines, additional incision lines for exposure, should the need arise.
Hemangioma affecting the nasal tip and columella subunit before (A) and after (B) surgical removal using the modified subunit approach. One procedure resulted in a normal contour. A small area of affected skin was left. The discoloration either fades on its own or can be treated with pulsed-dye laser.
One surgical procedure to correct hemangioma affecting the nasal tip, alar region, and nasal dorsum resulted in excellent nasal contours. A, Appearance before surgery. B, One week after surgery. C, Three years after surgery. A small section of affected skin was left behind during the procedure, which resulted in a slightly atrophic scar with some hyperpigmentation.
Hemangioma affecting the nasal tip, alar region, and paranasal area. A, Appearance before surgery. B, One week after surgery. C, Three years after additional procedures including further skin resurfacing of the scar and laser therapy of the residual hemangioma. Patient and parents were very satisfied with the overall outcome.
Once the incisions are made, the hemangioma is depressed, and with a scalpel blade held parallel with the skin, a plane is sought between the overlying skin and the underlying hemangioma. The skin flap is then carefully elevated using sharp dissection. The flap should be kept thin and consist of epidermis and dermis. Even in cases where there has been skin involvement, this plane of dissection should be sought. Elevation of the skin flap should continue until the entire hemangioma is exposed. At this point, the hemangioma can be carefully elevated off the domes and the medial crura of the lower lateral cartilages. The cartilages are then medialized with 1 or more dome sutures as well as a transfixion suture to narrow the columella. The skin flap is then redraped over the reconstructed nasal skeleton, and the excess skin is trimmed. Care should be taken not to disrupt the blood supply to this flap, which comes from the anterior ethmoidal arteries. If the hemangioma is compound and a plane of dissection is not obvious, a false plane should be created through the surface of the hemangioma. Any remaining redness can either be left to spontaneously fade or be treated using a pulsed-dye laser in the near future.
The patient characteristics are summarized in the Table. Of the 44 patients, 41 had focal hemangiomas, and 3 had segmental hemangiomas of the frontonasal or maxillary segment. The median age at the time of surgery was 15 months (range, 9 months to 12 years). The mean (SD) follow-up time was 41.6 (26.5) months.
Twenty-two patients had undergone 1 or more other treatments of their hemangioma before the present procedure (17, pulsed-dye laser therapy; 9, oral steroids; 2, intralesional steroids; and 1, vincristine). Nine patients were lost to follow-up after the postoperative period, most of whom were out-of-state patients.
Ten hemangiomas affected 1 subunit only (8, nasal tip; 1, alar; and 1, columella); 27 affected 2 subunits (7, nasal tip and alar; 19, nasal tip and columella; and 1, nasal tip and back of the nose); 5 affected 3 subunits (3, nasal tip, columella, and alar; 1, nasal tip, back of the nose, and paranasal; and 1, nasal tip, alar, and back of the nose); and 2 affected 4 subunits.
The surgical approaches were through an external rhinoplasty incision (13 patients), modified subunit incision (29 patients), and an elliptical incision (2 patients). Of all 44 patients, 24 did not need any additional treatment of the nose after the first and only surgical intervention; 6 needed 1 or more pulsed-dye laser treatments for the remaining reddish discoloration of the skin but no further surgery; and 14 patients needed at least a second surgical procedure.
Of the patients who underwent external rhinoplasty and who were still available for follow up (n = 10), 5 had a good outcome and 5 were improved. Four of the improved patients and one with a good outcome underwent a second surgical procedure. In 2 patients, residual hemangioma needed to be removed, and 1 patient experienced a slight regrowth of the hemangioma (this patient was 13 months old at the time of the first surgical procedure). Another patient needed a cartilage allograft of one of the medial crura of the lower lateral cartilages because the hemangioma had ulcerated and destroyed the columella. This patient had originally shown a good outcome after the first procedure but eventually needed a columella-lengthening procedure.
Of the 29 patients who underwent a modified subunit approach to their resection, 24 were available for follow-up. Of these, two-thirds had a very good or good result after 1 surgical procedure (8 very good and 8 good); 8 were improved. Eight patients needed further surgical treatment, and 3 underwent additional pulsed-dye laser therapy. The additional surgical procedures were to correct an unsatisfactory scar in 1 case (because the child had fallen on the nose shortly after the procedure), to remove a small portion of residual hemangioma in the nasolabial or nasofacial crease (n = 5), and to perform staged alar reconstruction (n = 2).
Of the 2 patients who underwent elliptical excision in the midline, 1 had an unsatisfactory result. This patient needed several reconstructive procedures of the columella and lateral alar cartilages and also additional pulsed-dye laser therapy.
Nasal hemangiomas are usually subcutaneous or mixed superficial plus subcutaneous (compound) lesions that occupy the space between the skin of the nasal tip and the lower lateral cartilages. As the hemangioma expands, it displaces the lower lateral cartilages laterally and deforms the nasal tip, thereby producing the classic bulbous hemangioma nasal tip. This is the most common deformity. Even if these hemangiomas involute spontaneously, the hemangioma is replaced with fibrofatty tissue. The displacement of the lower lateral cartilages is permanent, and the varying amount of residual fibrofatty tissue will leave a permanently bulbous nasal tip at the very least. Unfortunately, most nasal tip hemangiomas do not involute very well,15 and therefore, surgical correction becomes necessary. The hemangioma acts as a tissue expander, and after surgical removal, a large amount of excess skin is left. If this is not trimmed, the potential space left after the hemangioma has been removed will fill with serum that may organize and require further surgery.
Burget and Menick20 introduced the principle of subunit surgery in reconstructive surgery of the nose. Our surgical approach is based on the same subunit principle, but our incision line has been modified to allow better access to all of the nasal subunits as well as to allow trimming of the excess skin in 3 dimensions after the hemangioma has been removed (Figure 1). This is a major advantage of the subunit approach over other surgical approaches. Reconstructive approaches consist of approximating the domes of the lower lateral cartilages as well as the medial crura to achieve nasal projection and narrowing of the columella. Also, during most surgical procedures, the cartilaginous structures could be preserved. In all of our cases, it was possible to find the cartilages, and in no case were they ever involved with hemangioma. Furthermore, we avoid placing our incision in an anterior location,21,22 which we believe is much more readily visible.
We prefer to excise the bulk of the hemangioma but not necessarily all of the affected skin, since the skin coloration will eventually fade in the normal process of involution. Skin discoloration can also be treated with pulsed-dye laser in the months following surgery. A full-thickness graft or even flap would be much more noticeable than the affected skin after the red discoloration has faded. Great care must be taken in dissecting along the thin fibrous plane between skin, consisting of epidermis and dermal layer, and the hemangioma. The dermal flap is usually extremely thin and friable but needs to be preserved to cover the resulting defect.
The outcomes of patients treated with the modified subunit incision in our series was clearly better than those in the patients treated with open rhinoplasty or elliptical incision.
Our patient series is remarkable for the young age of our patients. The median age of the 44 patients was 15 months, and only 4 patients were older than 24 months at the time of surgery. Of these 4, 1 who underwent a modified subunit approach had a very good result, 2 had a good result, and 1 was improved by using the external rhinoplasty incision.
There are several advantages to performing surgery early. These include the psychosocial advantage of removing the stigma of a harlequin nose, Cyrano nose, or Pinocchio deformity before it becomes an issue (removal of the deformity before the patient becomes aware of being deformed) and the advantage of reduced scarring in young age.19 We therefore prefer surgical intervention before age 2 years, at which age the children develop self-consciousness and begin to sense the negative reactions of their peers. In our opinion there is little to be gained by postponing surgery beyond age 2 years because hemangiomas on the nasal tip usually do not involute very well, and even if they do, they leave behind fibrofatty tissue that eventually needs to be dealt with. On the other hand, surgery before age 1 year or in children who have had prolonged steroid treatment carries the risk of regrowth of any hemangioma tissue that may have been left behind during surgery. This may necessitate a second procedure. For these reasons we advocate surgical removal between ages 1 and 2 years.
In general, simple lesions that deform the nasal tip only slightly, but enough to warrant intervention, can be approached through an external rhinoplasty incision. Those that are larger and result in significant distortion of the tip will require removal of the lesion as well as resection of the excess skin, best accomplished through a modified subunit incision. A complex lesion involving more than one subunit is also best approached through a modified subunit incision because this approach will allow excellent exposure to adjacent areas and at the same time enable the surgeon to resect excess skin.
Our study proposes a modified surgical technique that we believe is superior to the ones we have used in earlier years. We have shown in a very young series of patients that removal of even large hemangiomas is possible with very good aesthetic results, even in young children. We therefore strongly advocate early surgical intervention.
Correspondence: Milton Waner, MD, Vascular and Birthmarks Institute of New York, 126 West 60th St, New York, NY 10023 (wanerMD@chpnet.org).
Accepted for Publication: February 27, 2008.
Author Contributions:Study concept and design: Waner and Scherer. Acquisition of data: Kastenbaum and Scherer. Analysis and interpretation of data: Scherer. Drafting of the manuscript: Kastenbaum and Scherer. Critical revision of the manuscript for important intellectual content: Waner. Statistical analysis: Scherer. Study supervision: Scherer.
Financial Disclosure: None reported.
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